La formación de intérpretes de lengua de signos: el caso de los CODA (hijos e hijas de padres sordos)

The lack of accessibility for deaf people leads their hearing sons and daughters, called CODA (Child Of Deaf Adults), to assume the language brokering role between the hearing and deaf world since their childhood. Sign language interpreting training programs have allowed anyone to enter this profession, and many CODAs have gone on to become professional interpreters. Through a descriptive phenomenological study, and after the analysis of twenty narratives of CODA who have participated in formal programs to become sign language interpreters in France and Spain, their specific needs in the classroom are revealed. The results of this research can benefit the field of sign language interpreting didactics by incorporating a CODA approach in the teaching design that favors the learning of all future interpreters.La falta de accesibilidad para las personas sordas conduce a que sus hijos e hijas oyentes, denominados CODA (Children Of Deaf Adults), asuman el rol de intermediación entre el mundo oyente y sordo desde su infancia. Los programas formativos de interpretación en lenguas signadas han permitido a cualquier persona acceder a esta profesión, y muchos CODA han pasado a desarrollar su labor de mediadores de manera profesional. Mediante un estudio fenomenológico de tipo descriptivo, y tras el análisis de veinte narrativas de CODA que han participado en programas reglados para ser intérpretes de lengua de signos en Francia y en España, se revela el conjunto de necesidades específicas de estos en las aulas. Los resultados de esta investigación pueden beneficiar al campo de la didáctica de la interpretación signada, incorporando un enfoque para los CODA en el diseño de la enseñanza que favorece el aprendizaje de todos los futuros intérpretes

Contribution of Energetically Reactive Surface Features to the Dissolution of CeO2 and ThO2 Analogues for Spent Nuclear Fuel Microstructures

In the safety case for the geological disposal of nuclear waste, the release of radioactivity from the repository is controlled by the dissolution of the spent fuel in groundwater. There remain several uncertainties associated with understanding spent fuel dissolution, including the contribution of energetically reactive surface sites to the dissolution rate. In this study, we investigate how surface features influence the dissolution rate of synthetic CeO2 and ThO2, spent nuclear fuel analogues that approximate as closely as possible the microstructure characteristics of fuel-grade UO2 but are not sensitive to changes in oxidation state of the cation. The morphology of grain boundaries (natural features) and surface facets (specimen preparation-induced features) was investigated during dissolution. The effects of surface polishing on dissolution rate were also investigated. We show that preferential dissolution occurs at grain boundaries, resulting in grain boundary decohesion and enhanced dissolution rates. A strong crystallographic control was exerted, with high misorientation angle grain boundaries retreating more rapidly than those with low misorientation angles, which may be due to the accommodation of defects in the grain boundary structure. The data from these simplified analogue systems support the hypothesis that grain boundaries play a role in the so-called “instant release fraction” of spent fuel, and should be carefully considered, in conjunction with other chemical effects, in safety performance assessements for the geological disposal of spent fuel. Surface facets formed during the sample annealing process also exhibited a strong crystallographic control and were found to dissolve rapidly on initial contact with dissolution medium. Defects and strain induced during sample polishing caused an overestimation of the dissolution rate, by up to 3 orders of magnitude

Transgenic expression of beta 1 antibody in brain neurons impairs age-dependent amyloid deposition in APP23 mice

Heterologous expression of the functional amyloid beta (A beta) antibody beta 1 in the central nervous system was engineered to maximize antibody exposure in the brain and assess the effects on A beta production and accumulation in these conditions. A single open reading frame encoding the heavy and light chains of beta 1 linked by the mouth and foot virus peptide 2A was expressed in brain neurons of transgenic mice. Two of the resulting BIN66 transgenic lines were crossed with APP23 mice, which develop severe central amyloidosis. Brain concentrations at steady-state 5 times greater than those found after peripheral beta 1 administration were obtained. Similar brain and plasma beta 1 concentrations indicated robust antibody efflux from the brain. In preplaque mice, beta 1 formed a complex with A beta that caused a modest A beta increase in brain and plasma. At 11 months of age, beta 1 expression reduced amyloid by 97% compared with age-matched APP23 mice. Interference of beta 1 with beta-secretase cleavage of amyloid precursor protein was relatively small. Our data suggest that severely impaired amyloid formation was primarily mediated by a complex of beta 1 with soluble A beta, which might have prevented A beta aggregation or favored transport out of the brain. (C) 2013 Elsevier Inc. All rights reserved

Carriers of the fragile X mental retardation 1 (FMR1) premutation allele present with increased levels of cytokine IL-10.

BACKGROUND: Fragile X-associated tremor/ataxia syndrome (FXTAS) is an inherited late-onset neurodegenerative disorder, characterized both by neurological and cognitive deficits. It is caused by the expansion of CGG repeats (55 to 200 repeats) in the noncoding region of the fragile X mental retardation 1 (FMR1) gene. Abnormal immunological patterns are often associated with neurodegenerative disorders and implicated in their etiology. We therefore investigated the immune status of FXTAS patients, which had not been assessed prior to this study. METHOD: Peripheral blood mononuclear cells (PBMCs) were collected from 15 asymptomatic FMR1 premutation carriers and 20 age-matched controls. Concentrations of three cytokines (IL-6, IL-8, IL-10) were measured in PBMC supernatants using ELISA assays. RESULTS: We found a significant increase in the concentration of the major anti-inflammatory cytokine IL-10 in supernatants of PBMCs derived from premutation carriers, when compared with controls (P = 0.019). This increase correlated significantly with the number of CGG repeats (P = 0.002). CONCLUSIONS: Elevated IL-10 levels were observed in all premutation carriers, before appearance of the classical neurological symptoms; therefore, IL-10 may be one of the early biomarkers of FXTAS

Social and clinical vulnerability in stroke and STEMI management during the COVID-19 pandemic: a registry-based study

Objective This study aims to evaluate whether the first wave of the COVID-19 pandemic resulted in a deterioration in the quality of care for socially and/or clinically vulnerable stroke and ST-segment elevation myocardial infarction (STEMI) patients.Design Two cohorts of STEMI and stroke patients in the Aquitaine neurocardiovascular registry.Setting Six emergency medical services, 30 emergency units, 14 hospitalisation units and 11 catheterisation laboratories in the Aquitaine region in France.Participants This study involved 9218 patients (6436 stroke and 2782 STEMI patients) in the neurocardiovascular registry from January 2019 to August 2020.Primary outcome measures Care management times in both cohorts: first medical contact-to-procedure time for the STEMI cohort and emergency unit admission-to-imaging time for the stroke cohort. Associations between social (deprivation index) and clinical (age >65 years, neurocardiovascular history) vulnerabilities and care management times were analysed using multivariate linear mixed models, with an interaction on the time period (pre-wave, per-wave and post-first COVID-19 wave).Results The first medical contact procedure time was longer for elderly (p<0.001) and ‘very socially disadvantaged’ (p=0.003) STEMI patients, with no interaction regarding the COVID-19 period (age, p=0.54; neurocardiovascular history, p=0.70; deprivation, p=0.64). We found no significant association between vulnerabilities and the admission imaging time for stroke patients, and no interaction with respect to the COVID-19 period (age, p=0.81; neurocardiovascular history, p=0.34; deprivation, p=0.95).Conclusions This study revealed pre-existing inequalities in care management times for vulnerable STEMI and stroke patients; however, these inequalities were neither accentuated nor reduced during the first COVID-19 wave. Measures implemented during the crisis did not alter the structured emergency pathway for these patients.Trial registration number NCT0497920

Low incidence of SARS-CoV-2, risk factors of mortality and the course of illness in the French national cohort of dialysis patients

International audienceThe aim of this study was to estimate the incidence of COVID-19 disease in the French national population of dialysis patients, their course of illness and to identify the risk factors associated with mortality. Our study included all patients on dialysis recorded in the French REIN Registry in April 2020. Clinical characteristics at last follow-up and the evolution of COVID-19 illness severity over time were recorded for diagnosed cases (either suspicious clinical symptoms, characteristic signs on the chest scan or a positive reverse transcription polymerase chain reaction) for SARS-CoV-2. A total of 1,621 infected patients were reported on the REIN registry from March 16th, 2020 to May 4th, 2020. Of these, 344 died. The prevalence of COVID-19 patients varied from less than 1% to 10% between regions. The probability of being a case was higher in males, patients with diabetes, those in need of assistance for transfer or treated at a self-care unit. Dialysis at home was associated with a lower probability of being infected as was being a smoker, a former smoker, having an active malignancy, or peripheral vascular disease. Mortality in diagnosed cases (21%) was associated with the same causes as in the general population. Higher age, hypoalbuminemia and the presence of an ischemic heart disease were statistically independently associated with a higher risk of death. Being treated at a selfcare unit was associated with a lower risk. Thus, our study showed a relatively low frequency of COVID-19 among dialysis patients contrary to what might have been assumed